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Kidney Week

Abstract: PO1550

Recurrence of Atypical Hemolytic Uremic Syndrome After Kidney Transplantation: A Prospective Cohort Study

Session Information

Category: Glomerular Diseases

  • 1203 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

  • Duineveld, Caroline, Radboudumc, Nijmegen, Gelderland, Netherlands
  • Bouwmeester, Romy N., Radboudumc, Nijmegen, Gelderland, Netherlands
  • Wijnsma, Kioa L., Radboudumc, Nijmegen, Gelderland, Netherlands
  • Van De Kar, Nicole, Radboudumc, Nijmegen, Gelderland, Netherlands
  • Wetzels, Jack F., Radboudumc, Nijmegen, Gelderland, Netherlands

Group or Team Name

  • CUREiHUS study group
Background

Since 2016, aHUS patients in the Netherlands are treated with a restrictive treatment protocol. Withdrawal of eculizumab is considered after a treatment period of three months. Furthermore, kidney transplantations in aHUS patients are performed without eculizumab prophylaxis, with initiation of eculizumab in case of post-transplant recurrence. This restrictive treatment protocol is monitored in the CUREiHUS study. Here, we present the CUREiHUS study results for kidney transplant patients.

Methods

All kidney transplant patients who received eculizumab therapy for a suspected aHUS recurrence, and who were included in the CUREiHUS study (after informed consent), were evaluated.

Results

In the period from January 2016 until October 2020 we included 15 (F 12, M 3; median age 42y, range 24-66) patients with suspected aHUS recurrence after kidney transplantation. Patients were classified as high (N=8) or moderate (N=7) recurrence risk. The time-interval to recurrence showed a bimodal distribution. Seven patients presented early after transplantation (median 3 m, range 0.3-8.8), with typical aHUS features: rapid eGFR loss and laboratory signs of TMA. Eight patients presented late (median 46m, range 18-69) after transplantation. Of these, 3 patients showed typical aHUS features, while in 5 patients no laboratory evidence of TMA was seen, and only a gradual eGFR loss. Treatment with eculizumab resulted in disappearance of TMA and improvement/stabilization of eGFR in 14 patients. Withdrawal of eculizumab was thus far proposed in 10 patients, and successful in only 5. Median follow-up after recurrence is 29 months (range 3-53 months). At last follow-up median eGFR was 32.0 ml/min/1.73m2 (range 7-80), considerably less than eGFR before recurrence (54.3 ml/min/1.73m2, range 22-103).

Conclusion

Patients with aHUS who develop recurrence after kidney transplantation do not fully recover kidney function. The major cause is treatment delay due to late recognition of disease recurrence in patients who present with a “creeping creatinine”. Discontinuation of eculizumab is often unsuccessful.